Prognostic factors and treatment effects on survival in erythroleukemia: A retrospective study of 134 cases

Abstract

A total of 134 cases of erythroleukemia (119 from the literature and 15 of the authors' patients) were reviewed in an attempt to correlate survival with age, sex, hepatomegaly, splenomegaly, lymphadenopathy, infection, or hemorrhagic complications at initial presentation; hemoglobin (Hgb), white blood cell (WBC) count, and platelet count; percent myeloblasts in the marrow at diagnosis; and treatment regimens employed. Statistical methods included single classification analysis of variance, nonparametric analysis of variance (Kruskal-Wallis method), contingency table analysis, and correlation coefficient determination for numerical data. No significant correlation between survival and age, sex, hepatomegaly, lymphadenopathy, infection, or hemorrhagic phenomena was found. Improved survival was noted in patients without splenomegaly, and in daunorubicin-treated cases in contrast to those treated with other chemotherapeutic agents. A positive correlation between survival and initial Hgb and WBC was also noted. Erythroleukemia complicating a chronic myeloproliferative disorder had a worse prognosis than de novo erythroleukemia.

Diagnosis and characterization of acute erythroleukemia subsets by determining the percentages of myeloblasts and proerythroblasts in 69 cases

“…Erythroleukemia constitutes approximately 3% of all cases of acute leukemia and is very rare in children [1][2][3]. DiGuglielmo's treatise on erythroleukemia (compiled by his sons after his death in l962) described two distinct clinical entities: DiGuglielmo syndrome, published originally in 1917, and DiGuglielmo disease, described in 1926 [4][5][6].…”

Hematopoietic stem cell transplantation for de novo erythroleukemia: a study of the European Group for Blood and Marrow Transplantation (EBMT)

“…Results with autologous transplantation were, in fact, not different from those of conventional chemotherapy alone, which showed median survival from 4 to 14 months. 8,[10][11][12][13][14][15] EL is a rare disease. This study is the only one giving actual information on HSCT in a large series of patients with EL.…”

“…8 EL is de novo for approximately half of patients, or secondary to myelodysplastic syndrome or chemotherapy and is frequently associated with multiple chromosomal abnormalities. 9 It has been reported that EL is less sensitive to chemotherapy than other AML subtypes, that median survival varies from 4 to 14 months, 8,[10][11][12][13][14][15] and that it has a tendency to transform into other AML subtypes. 9 Whereas the benefit of high-dose therapy with autologous or allogeneic hematopoietic stem cell transplantation (HSCT) for AML is now well established by several randomized studies comparing chemotherapy to autologous or allogeneic HSCT, [16][17][18][19][20] the real value of high-dose therapy with HSCT for EL is unknown.…”

Case 38-1997

“…Hypogranulated and pseudo-Pelger-Huët neutrophils are sometimes observed. 39 The clinical manifestations of erythroleukemia are similar to those of other acute leukemias, but there is a particular association with immunologic abnormalities. 40,41 A syndrome of synovitis and serositis with effusions has been described, as well as hypergammaglobulinemia and the presence of antierythrocytic autoantibodies, antinuclear autoantibodies, and rheumatoid factor.…”